Abstract 16152: The Mustard Operation for Transposition of the Great Arteries: Challenges During Adulthood
Background Survivors of the Mustard operation have largely transitioned to adulthood, yet their prognosis is uncertain.
Methods We investigated all 205 patients who underwent Mustard operation for d-TGA (1963-1991) and transitioned to our adult services. Outcomes were analyzed by parametric competing risks and repeated measures analysis of ECGs (2776), echos (1363), cardiopulmonary tests (321) and MRIs (288).
Results Attrition was constant (~1%/year); 20 years after transitioning to adulthood 79% were alive with a systemic RV circulation. Only 8 underwent transplantation. Intact ventricular septum was associated with improved late survival during adulthood (85% versus 69%, P=.05). The systemic RV gradually dilated over time (figure). This was associated with progressive decline in qualitative contractility (P<.0001) and calculated ejection fraction (P=.02). RV dilatation was particularly associated with VT (P=.004), but was unrelated to transposition morphology, surgical or bypass details. Tricuspid competence exhibited a gradual decline, but only 3 repairs/replacements have been undertaken. Congestive heart failure was common, affecting 43% of survivors at 20 years (figure). In all patients, VO2 max was less than predicted but remained constant. Older age at Mustard operation was associated with significantly lower late VO2 max (P<.0001). More than 50% survivors had undergone permanent pacemaker implantation by age 40. Baffle complications required surgical intervention in 11% at 20 years. Other serious complications were rare (endocarditis=2; late stroke=4; myocardial infarction=4).
Conclusions Gradual deteriorations in RV and tricuspid performance account for a high incidence of congestive heart failure in adults surviving Mustard operation. Nevertheless, very late survival is impressive, transplantation is rare and tricuspid or baffle interventions are infrequent. Inexorable RV dilatation will pose future management dilemmas.
- © 2011 by American Heart Association, Inc.